• S Peysson, N Vandenberghe, F Philit, C Vial, T Petitjean, F Bouhour, J Y Bayle, and E Broussolle.
• Faculté de Médecine Lyon Sud, Université Lyon 1, et Centre SLA, Hôpital Neurologique et Neurochirurgical, Hospices Civils de Lyon, Lyon, France. stephane.peysson@chu-lyon.fr
• Eur. Neurol. 2008 Jan 1;59(3-4):164-71.

Background/AimsThe involvement of respiratory muscles is a major predicting factor for survival in amyotrophic lateral sclerosis (ALS). Recent studies show that noninvasive ventilation (NIV) can relieve symptoms of alveolar hypoventilation. However, factors predicting survival in ALS patients when treated with NIV need to be clarified.MethodsWe conducted a retrospective study of 33 consecutive ALS patients receiving NIV. Ten patients had bulbar onset. We determined the median survivals from onset, diagnosis and initiation of NIV and factors predicting survival. Statistical analysis was performed using the Kaplan-Meier test and Cox proportional hazard models.ResultsThe median initial and maximal total uses of NIV were 10 and 14 h/24h. The overall median survival from ALS onset was 34.2 months and worsened with increasing age and bulbar onset of the disease. The median survival from initiation of NIV was 8.4 months and was significantly poorer in patients with advanced age or with airway mucus accumulation. Survival from initiation of NIV was not influenced by respiratory parameters or bulbar symptoms.ConclusionAdvanced age at diagnosis and airway mucus accumulation represent poorer prognostic factors of ALS patients treated with NIV. NIV is a helpful treatment of sleep-disordered breathing, including patients with bulbar involvement.Copyright 2008 S. Karger AG, Basel.

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